Oro-facial presentations of CKD

The Forgotten Story

By

Dr. Ahmed Mohammed Abd El Wahab

Lecturer of internal medicine-NephrologyMansoura Faculty of medicine

Types of facial skeletal changes

1. Osteitis fibrosa cystica & brown tumours (commonest)

2. Fibrous dysplasia like lesion3. Leontiasis ossea (rarest)

Leontiasis Ossea

It is important to recognize features of uremic leontiasis ossea, as it may result in:

• Life-threatening upper airway obstruction

•Compressive cranial neuropathy.

Sagliker Syndrome(The Big Failure)

Introduced in 2004 in patients with ESKD and severe SHPT

This syndrome describes maxillary and mandibular deformities, dental abnormalities, benign soft tissue tumors in mouth, and various kinds of skeletal changes including short stature and fingertip abnormalities.

Fibrous Dysplasia

OFC(brown tumour)

 

Drugs1. Bisphosphonates-----> Avascular Jaw necrosis

Criteria to diagnose:

1. The patient possesses an area of exposed bone in the jaw persisting for more than 8 weeks,

2. The patient must present with no history of radiation therapy to the head and neck,

3. The patient must be taking or have taken bisphosphonate medication.

Contd,.

2. CNIs, nifedipine -----> Gingival hyperplasia(>3m ttt & children)

3.Anticoagulants----> Gingival bleeding

4.Diuretics, BBs----> lichenoid lesions

International evaluation of unrecognizably uglifying human faces in late and severe secondary hyperparathyroidism in chronic kidney disease. Sagliker syndrome. A unique catastrophic entity, cytogenetic studies for chromosomal abnormalities, calcium-sensing receptor gene and GNAS1 mutations. Striking and promising missense mutations on the GNAS1 gene exons 1, 4, 10. J Ren Nutr. 2012 Jan;22(1):157-61.

Gene Mutations in Chronic Kidney Disease Patients With Secondary Hyperparathyroidism and Sagliker SyndromeMarch 2015Volume 25, Issue 2, Pages 176–186, Journal of

renal nutrition

THANK YOU